Table 1.

Summary of reported cases of pneumatosis intestinalis associated with juvenile dermatomyositis.

	Onset age of PI	Period from JDM onset to PI onset	Treatment before PI onset	Manifestations of PI	Image findings and clinical diagnosis	Disease activity of JDM at PI onset	Clinical course of PI	Outcome
1 [13]	4.8, female	1 Y	GCs	Abdominal pain, diarrhea, constipation, and movable mass in the left lower quadrant	Cystoid gas collection in the mid transverse colon and splenic flexure	Calcinosis universalis was noted	Pneumatosis persisted for 25 months	Survived
2 [14]	8.5, female	3 Y	GCs, AZP, CY, and MTX	Abdominal pain and abdominal distention	Gas-filled hepatic flexure of colon and extraluminal gas	Refractory myositis and skin manifestations with disseminated subcutaneous calcification	Abdominal manifestations remitted during the next 10 days with PSL and MTX. Intramural gas persisted for four months	Survived
3 [15]	12, female	3 Y	None	Dysphagia	Cystoid gas collection	Noted myositis and dermatitis	Treated with oral GCs	Survived
4 [10]	8.5, female	1 Mo	GCs and MTX	Abdominal pain, vomiting, diarrhea, and fever	Perforation and peritonitis	Increased muscle weakness, rash, and rising muscle enzyme values recurred	Died 6 weeks after the development of PI owing to complications of perforations, peritonitis, and candida sepsis	Died
5 [11]	11, female	3 Mo	GCs and CY	Abdominal pain, bilious emesis, and fever	Extensive extraluminal gas collection in the right abdomen and flank. Peritonitis, retroperitoneal abscess, and duodenal perforation were found at laparotomy	Became bedridden because of progressive muscle weakness from JDM onset	Discharged 8.5 months after admission over multiple episodes of sepsis and 8 laparotomies	Survived
6 [11]	15, male	2 Y	GCs	Abdominal discomfort, pain, vomiting, fever, and hematemesis	Following appendicitis and appendectomy, PI occurred with duodenal perforation and peritonitis	Exacerbated muscle weakness two months previously	Died of perforation, sepsis and multiple organ failure on the 21st hospital day despite four laparotomies	Died
7 [12]	7, female	3 Mo	GCs and MTX	Abdominal pain	Intramural air in the ascending and transverse colon	Prominent skin rash and vascular ulcers in the axillar, minimal proximal muscle weakness	Treated with intravenous antibiotics and parental nutrition. Clinical improvement evident after a week	Survived
8 [8]	8, female	1 Mo	GCs, MTX, and mPSL pulse	Cough and abdominal distention	CT showed extensive PI in the large colon	Increased weakness and a vasculitic ulcer on upper eyelid and in the nare	Treated with intravenous antibiotics and a short period of bowel rest	Survived
Present case	16, female	6 Y	GCs	Abdominal pain and abdominal distention	Extensive gas-filled colon and extraluminal gas above colon	Intermittently worsened skin manifestations	Complicated with strangulated obstruction and large intestine resection and colostomy were performed	Survived

PI: pneumatosis intestinalis, JDM: juvenile dermatomyositis, Mo: month(s), Y: year(s), GCs: glucocorticoids, PSL: prednisolone, CY: cyclophosphamide, AZP: azathioprine, MTX: methotrexate, and mPSL: methylprednisolone.